ZHENG Zhi-dong, FENG Kai. Department of Neurology, Shunyi Hospital, Beijing 101300, China

[ Abstract ]  Objective: To analyze the clinical characteristics, imaging and prognosis of progressive supranuclear palsy (PSP). Methods: The clinical data and prognosis of 7 cases of PSP were collected. Results: The onset age of 7 patients was 56-74 years, with a median of 66 years, The initial symptoms was early walking instability with falling, 4 patients showed symptom of dizziness,2 showed unexplained anxiety and depression. Typical vertical ophthalmoplegia and typical MRI imaging changes appeared in all cases within 4 and 5 years respectively. According to the latest clinical diagnostic criteria, 5 cases were diagnosed as probable PSP-RS, 2 cases were PSP-P. Conclusion: The typical symptoms of progressive supranuclear palsy are mostly unsteady walking and abnormal posture accompanied by falling, bradykinesia and rigidity with axial predominance with levodopa resistance. Patients maybe visited the ophthalmology or psychiatric department for the first time with the symptoms such as anxiety, depression, dizziness, and vision loss. The typical vertical ophthalmoplegia and typical images of midbrain atrophy may appear as the disease progresses, and the prognosis is poor.

7例进行性核上性麻痹临床首发症状特点

郑志东  冯  凯 

北京顺义区医院神经内科

2020年10月  第3卷  第4期  文章编号  2096-5516 (2020) 04-0293-05  doi: 10.3969/j.issn.2096-5516.2020.04.007

[ 摘  要 ]  目的：分析进行性核上性麻痹的临床特征、影像学表现及预后。方法：通过观察及随访7例临床诊断进行性核上性麻痹患者，收集其相关临床资料，并随访患者生存情况。结果：7例进行性核上性麻痹患者，发病年龄56~74岁，中位数66岁，4例患者早期出现头晕，2例存在不明原因的焦虑抑郁，其余患者早期以走路不稳伴摔倒， 4年内出现典型的垂直性眼肌麻痹，5年左右出现头颅核磁提示中脑被盖部萎缩，中脑前后径变小，呈现该病典型改变，多数患者约6~8年需他人帮助生活，依据最新的临床诊断标准，5例诊断为很可能PSP经典型（PSP-RS），2例为PSP帕金森综合征型（PSP-P）。结论：进行性核上性麻痹典型症状多有走路不稳姿势异常伴摔倒，多存在轴性为主姿势异常，多表现为翻身及转身困难，美多巴治疗效果欠佳，多数患者早期有头晕、焦虑、抑郁状态改变，偶有视力改变，可能以视力、精神因素第一时间就诊于眼科或精神科，随着疾病进展，均可出现典型的垂直性眼肌麻痹及典型影像学改变，总体预后不佳。